Preoperative and postoperative evaluation of cerebral perfusion and vasodilatory capacity with 99mTc-HMPAO SPECT and acetazolamide in childhood Moyamoya disease.

Background And Purpose: The results of long-term follow-up studies of cerebral perfusion and vasodilatory capacity following administration of acetazolamide after serial vascular reconstructions in 25 patients with childhood moyamoya disease are reported.

Methods: Cerebral perfusion was measured with 99mTc-hexamethylpropyleneamine oxime single-photon emission CT before and after IV administration of 10 mg/kg acetazolamide, which was performed both before and after vascular reconstruction by superficial temporal artery-middle cerebral artery anastomosis and encephalomyosynangiosis (first and second operations) and/or omental transplantation to the brain (third operation).

Results: Follow-up periods ranged between 12 and 24 months (mean +/- SD, 18.5 +/- 3.2 months) after the first operation. Repetitive transient ischemic attacks disappeared completely after serial vascular reconstructions in all patients. Before the first operation, cerebral perfusion in the territory of the middle cerebral artery on the side of initial operation was 83.9 +/- 4.7% and was significantly lower than that in the contralateral side (88.3 +/- 4.9%, n = 25; P < .0001, paired t test). Vasodilatory capacity on the side of the first operation was -18.4 +/- 2.5% and that on the contralateral side -14.4 +/- 2.1%. The former value was significantly lower than the latter value (n = 25; P < .0001, paired t test). After the first operation, cerebral perfusion and vasodilatory capacity on the side of initial operation were markedly improved, to 87.8 +/- 4.5% and -14.7 +/- 2.7%, respectively (n = 25; P < .0001, both cases, paired t test). Before the second operation, cerebral perfusion and vasodilatory capacity on the side of the second operation were 76.6 +/- 4.1% and -20.1 +/- 1.9%, respectively, and significantly lower than those before the first operation (n = 25; P < .0001, both cases, paired t test). Eight patients subsequently required bifrontal omental transplantation for repetitive paraparetic transient ischemic attacks after the second operation; they had low cerebral perfusion and vasodilatory capacity bilaterally in the territories of the anterior cerebral arteries (72.4 +/- 2.7% and -18.6 +/- 1.7%, respectively). After omental transplantation, both were significantly increased, to 81.9 +/- 3.4% and -11.8 +/- 1.9%, respectively (n = 25; P < .0001, both cases, paired t test).

Conclusions: Hemodynamic compromise existed in patients with childhood moyamoya disease and was a major cause of development of ischemic symptoms. Regions in which hemodynamic compromise was present could be determined by measuring regional cerebral perfusion and vasodilatory capacity.